CASE REPORT Bladder Malakoplakia Simulating Neoplasm in a Young Girl: Report of a Case and Review of Literature Seyed Mohammad-Reza Rabani1*, Seyed Hossein Rabani2 Associate Professor of Urology and Renal TX; Beheshti Teaching Hospital; Yasuj University of Medical Scienc- es; Yusuj, Iran. Tel: +989177411389. E mail: smrrabani@yahoo.com. Rabani.smr@yums.ac.ir. Medical Researcher, Shahid Beheshti University of Medical Sciences. +989123254037. Rabani.md@gmail.com. *Correspondence: Associate Professor of Urology and Renal TX; Beheshti Teaching Hospital; Yasuj University of Medical Sciences; Yusuj, Iran. Tel: +989177411389. E mail: smrrabani@yahoo.com. Rabani.smr@yums.ac.ir. Received February 2018 & Accepted August 2018 Malakoplakia is a granulomatous disorder caused by infectious process. It was described by Von Hanseman in 1901 for the first time and then by Michaelis and Gutman in 1902. Although the most frequent site of involvement is genitourinary tract, various organs have been reported to be affected. The peak age incidence is about 50 years and it is rare in childhood. In this paper we report a case of bladder malakoplakia which to our knowledge is the youngest with isolated bladder malakoplakia that has been reported. Keywords: bladder; children; malakoplakia; neoplasm; pediatrics INTRODUCTION Malakoplakia is a rare granulomatous disease of infectious etiology that most commonly is found in genitouri-nary tract. It is commonly observed in immunocompromised patients. Depending on the organ involvement, the patients may present in a myriad of ways and causing a huge diagnostic challenge. Malakoplakia is microscop- ically characterized by a collection of large mononuclear cells with abundant cytoplasm(1). These cells are called Hanseman macrophages and are full of calcium and iron-laden lysosomal material that are known as Michaelis – Gutman bodies. CASE REPORT The patient was a 20-month-old girl that was brought for voiding dysfunction and repeated urinary tract infections and E. coli growth in urine culture. Urinalysis revealed pyuria. Blood profile, renal, and liver function tests were normal. Abdominal and pelvic ultrasound and C.T. Scan showed a mass in anterior part of the right lateral wall of the bladder, about 4 by 4 by 2.5 cm in size with suggestion for possibility of central necrosis (Figure 1). Cystos- copy showed a bladder mass, but biopsy was not informative and was suggestive for chronic cystitis. Exploration was done from a low-midline, retroperitoneal incision as the clinical diagnosis was a malignant neoplasm and the mass was resected with enough free margins (partial cystectomy). Histopathology report (Figure 2) suggested malakoplakia. The patient received trimethoprim-sulfamethoxazole for 3 months after partial cystectomy and her Figure 1. C.T.Scan of the pelvis. Figure 2. Histopathology of the bladder mass suggestive for Malakoplakia. 2(a): Michaelis-gutmann body. 2(b): Michae- lis-gutmann body. 2(c): Michaelis-gutmann body. 2(d): Michae- lis-gutmann body. 2(e): Michaelis-gutmann body Urology Journal/Vol 16 No. 6/ November-December2019/ pp. 614-615. [DOI: 10.22037/uj.v0i0.4428] Vol 16 No 06 November-December2019 500 9 years post- operative period was uneventful. DISCUSSION Malakoplakia is usually seen in immunocompromized patients, but it can also be seen in immunocompetent individuals. Although the most common site of involve- ment is genitourinary tract, other common sites are gas- trointestinal tract and retroperitoneum, but it can be seen everywhere in the body(2). It is more common in males except for malakoplakia of genitourinary system that is more common in females. The peak age incidence is about 50 years and it is rare in childhood. The typical lesion of malakoplakia is grossly characterized by a soft yellow-brown mass or plaque with central ulceration and peripheral hyperemia. A patient with malakoplakia may present with a range of findings, but the standard criterion for diagnosis is pathologic evaluation. The pathologic findings are caused by defects in phagocytic degradative function of histiocytes in response to gram negative coliforms (E. coli or Proteus) that results in a chronic inflammatory process, followed by intracellular deposition of Calcium and Iron, a pattern that is known as Michaelis - Guttmann bodies. Large macrophages or von Hansemann cells with a variable inflammatory cells consisting mainly of lymphocytes, plasma cells and neutrophils are microscopic findings in malakopla- kia. Although there is not a definite cause and effect re- lationship between coliforms and malakoplakia, many studies have shown an incidence of 89% to 93% coli- form infections in patients with malakoplakia(3,4). Kajbafzadeh and Baharnoori have reported a case of re- nal malakoplakia simulating neoplasm in a 10-year-old boy suffering from fever and headache for 20 days ac- companied with poor condition and cachexia. An open biopsy of the mass was suggestive for malakoplakia and a trial treatment with bethanechol chloride, 12.5 mg three times daily, trimethoprim-sulfamethoxazole, one adult tablet per 12 hours, and ascorbic acid, 500 mg three times daily for 21 days managed the disease without surgical intervention(5). Amar Shah and Harish Chandran reported a case of malakoplakia presenting as multiple bladder polyps in an 11-year-old boy with no response to long-term anti- biotic treatment, they performed surgical excision of the polyps and resolved his problem(6). Also they proposed surgical excision as an alternative form of management of this rare lesion. Surgical excision as an alternative treatment also has been suggested for very large lesions witch complete eradication of them may be impossible by medical therapy alone(7). Kuldeep and coworkers reported spontaneous perforation of the bladder in a 9-year-old female with coexistence of xanthogranu- lomatous cystitis with malakoplakia(8). Raghavaiah and coworkers have reported a case of nephrogenic adeno- ma of urinary bladder associated with malakoplakia in a 12-year-old female child, associated with recurrent Es- cherichia coli urinary tract infection, but to our knowl- edge our patient is the youngest with isolated bladder malakoplakia that has been reported.(9) REFERENCES 1. Damjanov I, Katz SM. Malakoplakia. Pathol Annu. 1981;16:103-26. 2. Curran FT. Malakoplakia of the bladder. Br J Urol. 1987;59:559-63. 3. Stanton MJ, Maxted W. Malacoplakia: a study of the literature and current concepts of pathogenesis, diagnosis and treatment. J Urol. 1981;125:139-46. 4. Deridder PA, Koff SA, Gikas PW, Heidelberger KP. Renal malacoplakia. J Urol. 1977;117:428-32. 5. Kajbafzadeh A, Baharnoori M. Renal malakoplakia simulating neoplasm in a child: successful medical management. Urol J. 2004;1:218-20. 6. Shah A, Chandran H. Malakoplakia of bladder in childhood. Pediatr Surg Int. 2005;21:113-5. 7. Ammani A, Ghadouane M, Janane A, Moufid K, Ameur A, Abbar MJAJoU. Pseudotumoral malacoplakia of the bladder. 2009;15:107-10. 8. Sharma K, Singh V, Gupta S, Sankhwar S. Xanthogranulomatous cystitis with malacoplakia, leading to spontaneous intraperitoneal perforation of the urinary bladder in a 9-year-old girl. BMJ Case Rep. 2015;2015. 9. Raghavaiah NV, Noe HN, Parham DM, Murphy WM. Nephrogenic adenoma of urinary bladder associated with malakoplakia. Urology. 1980;15:190-3. Bladder malakoplakia-Rabani et al. Vol 16 No 06 November-December2019 615